Cardiology Fup, 3 weeks post-op, Cont'd Valve changes.

Another week (3 weeks post-heart surgery), another Cardiac fup, Labs and Echo. I've come to the conclusion good news doesn't follow me to much atleast medical wise but then I can't say I am ever to surprised. I do believe 'one day at a time' is the best way to handle when you do get bad news, it works for me anyways. I know for others they have other ways of coping and nothing wrong with that either.

This article seems to do a good job of explaining what was done during the Konno Procedure/AVR surgery and what has happened including the "it shouldn't happen in adults" which if nothing else and perhaps sad humor on my part made me laugh (see below).

Aortic Stenosis - Konno Procedure This procedure is performed in cases of Aortic Stenosis when the left ventricular outflow tract is stenotic in addition to the aortic valve itself. It involves the replacement of the aortic valve and the widening of the ventricular septum in the region of the valve with a patch (see illustration). The aortic valve is replaced with either a mechanical valve (Konno-Rastan Procedure), an aortic homograft (human aortic valve), or with the patient's own pulmonary valve. In the last case, the valve may grow and renew itself over time. However, the first two types of replacement valve will eventually need to be replaced as the function over time decreases. After the Konno Procedure, the possibility of renewed narrowing of the left ventricular outflow tract (LVOT) exists and the patient will need regular monitoring. The development of a new systolic murmur or the intensification of a soft murmur is an indication that the LVOT should be investigated through echocardiography. The aortic valve itself also needs to be monitored to guard against progressive aortic insufficiency. If this becomes significant, reoperation will be necessary. However, significant aortic insufficiency is uncommon in the adult patient and mild insufficiency is well-tolerated and generally remains stable for long periods. Most surgically implanted mechanical valves will last 10-20 years before they wear out, become obstructed, or lose efficiency. When their function becomes impaired for any of these reasons, replacement becomes necessary

The good news is I can start cardiac rehab, so the NP is helping get that set up and coordinated. If I do well with this it shows the team I am recovering well and handling the changes to the new valve ok. If I am symptomatic during it gives them a good idea of how to proceed I guess to.
 The bad news is the Echo shows the new aortic valve has went from the post-surgery leaks being mild and concerning to the leaks being moderate (change from last weeks Echo to this weeks repeated Echo) at 3 wks post-op. To quote Dr.E  "this is more concerning" as well as "disappointing"  but they'll con't to watch closely, keeping pre-AVR meds the same for now and repeat the Echo again in 2 weeks with fup after. If no changes we likely would not have to do anything w the new valve but con't to watch it but if it continues to worsen then we would have to go back in and replace this aortic valve with a new one meaning another open heart surgery, balancing the steroids, anesthesia, etc. The upside of a downside to that would be we wouldn't have to repeat the aortic root enlargement (konno procedure) which made this last OHS that much longer and more complicated. I'd really like to not have to do anything heart related! They know the leaks are coming from the aortic root enlargement and subsequent patch and believe a stitch/stitches have come loose leading to the leaking. Had they not enlarged this aortic root and annulus they would have had to use a pediatric size valve though which in turn would have posed it's own issues and would have been more likely to cause less overall success with the surgery and heart long-term. I just would hope this settles out soon enough as i'd really like for there not to be a delay in our being able to place the Thoracic shunt back in to the pleural space sooner rather than later (8-12 wks post-op per Cardiology)! Not that I want any surgery but that shunt draining elsewhere makes a significant difference. I fup w Dr.Bragg, Neurosurgery next week so will be curious what she has to say.

 Today's labs show the Coumadin level  cont's to be to low so this was adjusted from 4mgs 5 days a week and 6mgs 2 days a week to 6 mgs 5 days a week and 4 mgs 2 days a week with repeat INR again before the fup Appt in 2 weeks.

Dr.E said to me today something along the lines of  "if it was going to happen to someone I guess I am not surprised it was you", meaning w the MPS and other surgeries i've sailed through many-a surgeries only then to have complications afterward (shunts for instance).  Probably isn't funny and he didn't really mean it to be but in the face of bad news that statement kind of made me laugh. What else are ya going to do, right? Not worth stressing over to me as to much can change in an instant anyways. He did say given how worried he was before the heart surgery and my being high-risk + how hard he thought the surgery/post-op would be that he was happy I sailed through this post-op and recovery as well as I did! I agree!! He has admitted he's disappointed and worried, I figure i'll leave that to him (and my Mom whom I know this drives crazy) and like I said above I figure I can't change it anyways so it is in God's hands and i'll worry about what I can influence ie other things. =)

On a side note we had our 2nd (my first) Sunday School meeting of the year, planning the 1st day (we are going to do a group-all classes activity one sunday a month vs every sunday in our individual classrooms) as well as going over our information for canvassing in a few wks which just means passing out flyers/brochures related to SS. Last we went over a bit of the SS Conference in mid-Sept. After a long afternoon in Milw I was already tired but still a good meeting.
Thanks for stopping by,

Erica

Cardiology Fup update, An Anesthesia Provider-Summary option

Yesterday was the 1st fup with the Cardiology Team since I got released last Friday including Labs, Xray, repeat Echo (3rd regular one since the surgery) and fup w the Cardiologist. All seems to con't to be going ok with the new post-surgery VSD and 2 aortic valve leaks being stable and INR (blood thinner level) was just a little low so that med dose was adjusted accordingly. Their main worry cont's it seems to be is there a infection (bacterial endocarditis) but I have no symptoms of this so I think they just are watching closely to be sure.
The next follow up appt will continue to be in 1 week with Labs and repeat Echo followed by seeing the Cardiologist again. Otherwise the only change was to the dose of Coumadin as level was somewhat to low, this just went up to 6mgs 2 days a week and 4mgs 5 days a week. This level going up and down isn't that unusual in the beginning as ones body adjusts to the med and blood levels being thinned.

Otherwise i've had to change around a few appts because of these Cardiology fups with seeing Pain Mngmt dr next week and then Neurosurgery the following week. We have to wait 8-12 weeks to replace the Thoracic shunt back in to the pleural space but Dr.Bragg had wanted a fup appt about a month after I last saw her or when I was feeling well enough from the heart surgery. I think one she may want to check the shunt settings (?) and 2 I imagine we'll begin to look at placing the shunt back ie a timeframe and plan which I imagine will be sometime in Oct. though not certain. Otherwise just kind of bored and looking fwd to being able to drive again!!!!

Below I've posted a summary letter I received from the Anesthesiologist at CHW who did the heart surgery and the TEE as she emailed me this the other day as a means for future new providers/surgeries to be aware of what they will be dealing with. I only rarely post this kind of thing but have been thinking about this and really think it is a good example of something MPS Families/Adults might want to consider requesting from their Anesthesiologist as it can be helpful, is well laid out and explains all airway issues this dr ran in to very well. As I think I posted in one of the previous updates this dr commented to me how un-helpful the actual Anesthesiol. notes from UW where in preparing her for what she would see and so she asked me if writing something like this would be helpful in order for future, new drs to not minimize the issues ahead of time. I will leave the providers full name off the end.


August 3, 2013

To Whom It May Concern:

Last month, I had thAuguste pleasure of providing anesthesia to Erica Thiel for aortic valve replacemen and subsequent TEE prior to her discharge.  As you are aware, she has Hurler Scheie type 1, which is commonly associated with airway challenges during anesthesia, becoming progressively worse as the patient gets older.  

Erica has a number of features on physical examination that suggest intubation may be challenging: limited extension of the cervical spine due to her disease as well as cervical fusion from C2-4; variable degree of mouth opening, and Mallampati III. However she has normal jaw contour and generous thyromental distance.  

Her face is somewhat narrow and the #4 mask provides a much better fit than does the #5 adult mask. She is easily ventilated by mask with or without muscle relaxation.

It should not come as a surprise that direct laryngoscopy was not successful. The Macintosh #3 blade would not advance to the base of the tongue. A glidescope provided visualization of the posterior aspect of her larynx and vocal cords but I was not able to advance the endotracheal tube into the hypopharynx due to her small mouth size and limited opening under anesthesia.  Nasal fiberoptic intubation was successful after dilation with 26 and 28 nasopharyngeal airways, although the insertion of same resulted in nasopharyngeal bleeding.  In addition, once anesthetized, she had copious oral secretions.

Following her aortic valve replacement, she remained intubated to avoid potential airway difficulties in the setting of emergency return to the operating room for bleeding or other surgical complications.

She underwent TEE prior to discharge, and tolerated the procedure well. She was progressively sedated with midazolam, fentanyl and ketamine without any airway challenges throughout the case.

She has adrenal insufficiency and thought that perhaps her dizziness and strange sensation following the procedure might have been due to the lack of a stress dose of steroids, which I did not administer because I did not think that the echocardiogram would be sufficiently stressful to her physiology.  

Erica is keenly aware of her medical issues and deals with them quite effectively.  She has requested that I provider her with this information hoping that it may be useful to other providers who are unfamiliar with her history.

Sincerely,

 

Thanks for stopping by,

 

Erica